YO Clinical Case: Mysterious vision loss in a teenager

A 14 year old patient presented with sudden vision loss in the right eye. There was a central negative scotoma in his right eye for 10 days.

Examination revealed:

VOD: Count fingers at 50 cm

VOS: 20/20

Slit lamp examination OO: negative

Pupils: isochoric, isocyclic and reactive to light. Direct and consensual pupillary light reflexes were regular and there were no abnormalities found in ocular motility and convergence

IOP OO: 14 mmHg (pneumotonometry)

The differential diagnoses were:

– Coats’ disease: occurs at an earlier age

– Cat-scratch disease: the patient has a cat and reports that he was scratched a few weeks prior to the vision loss

– Other infectious neuroretinites: Syphilis, lyme disease, rocky mountain spotted fever, toxoplasmosis, toxocariasis, histoplasmosis, and leptospirosis

– Retinal vasculitis (infectious or autoimmune diseases)

– Retinal macroaneurysm: not associated with papillitis and vasculitis

– Eales disease: occurs at an older age; absence of papillitis

Further information:

Cat-scratch disease

Blood tests: negative

Bartonella henselae: absence of antibodies and general symptoms (lymphadenopathy, fever, decreased appetite, headache, chills, muscular pains)

Other infectious diseases

Blood tests: negative for antibodies against syphilis, cytomegalovirus, toxoplasmosis, tuberculosis, borrelia, HIV etc.

Antibodies against Epstein Barr Virus were found.

Retinal vasculitis

• Urinalysis: presence of glycosuria, increased creatinuria, potassium and magnesium
• Suspected retinitis from autoimmune interstitial tubular nephritis
• Nephrological visit: negative
• Autoantibodies for autoimmune diseases: negative (Anti-DNA, ANCA, ANA, FR, AMA, ENA, PCR)

          No signs of vitritis

While awaiting fluorangiography, empirical therapy for an Epstein Barr Virus infection was started in association with corticosteroid therapy to reduce retinal and optic disk inflammation:

– Acyclovir 400 mg 3 times per day

– Prednisone 1 mg/kg

Fluorescein Angiography showed neuroretinitis, retinal macroaneurysm with exudation, vasculitis, IRMA and  ischemia in peripheral vessels.

A diagnosis of exclusion was made:

Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis Syndrome (IRVAN)

IRVAN

• First defined in 1983 by Kincaid and Schatz
• Unknown etiology
• Three major fundus findings: multiple aneurysmal dilatations, retinal vasculitis and neuroretinitis in arterial bifurcation
• Three minor fundus findings: peripheral capillary perfusion disorder, retinal neovascularization and macular exudation
• Very rare disorder that is seen in young people
• Diagnosis of exclusion with no specific tests

Karasu B et al. The fluorescein angiographic photodiagnosis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: Outcome of combined therapy. Photodiagnosis Photodyn Ther. 2019

Chong YJ et al. Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging. Graefes Arch Clin Exp Ophthalmol. 2019

TREATMENT

• Oral therapy with Prednisone 1 mg/kg
• Intravitreal Dexamethasone Implant combined with an Aflibercept Injection in the same session – First therapy
• Focal laser treatment to the macroaneurysm – 2 weeks after the Dexamethasone Implant
• Sub-threshold micropulse laser treatment to the macula – 2 weeks after the Dexamethasone Implant (in the same session as focal laser treatment)
• Panretinal Photocoagulation to the peripheral areas of non perfusion – one month after the Dexamethasone Implant

At the latest follow-up the patient presented BCVA 20/40 in the right eye, with a central macular thickness of 464 microns at OCT scan

No side effects were noted except a mild IOP rise treated with topical therapy.

CONCLUSION

• IRVAN is a diagnosis of exclusion with no specific tests
• The progression of IRVAN can vary greatly, despite aggressive treatment with PRP, intravitreal steroids and anti-VEGF agents
• The therapeutic approach must be customized based on the severity of the clinical findings: Intravitreal Dexamethasone Implant is effective in treating retinitis, papillitis and inflammatory exudation
• Multiple imaging modalities including Fluorescein angiography, OCT and OCT-angiography provide valuable information for disease monitoring and progression
• In our case, the patient will undergo a new Intravitreal Dexamethasone Implant